Abstract
Introduction: Improved diagnosis and awareness have resulted in global increases in the incidence and prevalence of transthyretin amyloid cardiomyopathy (ATTR-CM). However, current US epidemiology data are lacking. Aims: To estimate the prevalence and incidence of ATTR-CM in a large US population and describe characteristics of patients with ATTR-CM. Methods: Data from 2004–2022 were extracted from TriNetX, a US database of EMRs covering in- and outpatient data. An algorithm using ICD-9/10 codes and RxNorm codes for clinical drugs (amyloidosis + cardiac manifestation – light chain amyloidosis) was used to identify patients with ATTR-CM. Included were adults ≥ 18 years old at index date (first ATTR-CM diagnosis per the algorithm) with ≥ 1 year of database history. The incidence and prevalence of ATTR-CM were assessed annually. Demographic characteristics at index and ATTR-CM-related comorbidities any time pre-index were assessed in cross-sectional cohorts over time. Results: In total, 12 209 patients were identified (2004–08, n = 332; 2009–13, n = 913; 2014–18, n = 3392; 2019–22, n = 7572). The incidence and prevalence of ATTR-CM per 100 000 people increased over time (incidence, 2004: 3.7; 2022: 11.5; prevalence, 2004: 5.1; 2022: 35.1) ( Figure ). Across cohorts, approximately 40% of patients were women, and 20% were Black. Median age increased over time (2004–08: 65 years; 2019–22: 74 years). In the 2019–22 cohort, the cardiac manifestations of ATTR-CM were cardiomyopathy (56.3%), atrial fibrillation (56.1%), and heart failure (20.9%). The proportion of patients with ATTR-CM-related comorbidities increased over time (2004–08 vs 2019–22: polyneuropathy, 15.1 vs 19.0%; carpal tunnel syndrome, 10.5 vs 13.4%; lumbar spinal stenosis, 4.2 vs 13.3%; aortic stenosis, 9.3 vs 13.2%). Conclusion: In this US population, the incidence and prevalence of ATTR-CM diagnoses increased over 19 years. Age at diagnosis and ATTR-CM comorbidity burden increased during this period.
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